Anomalous origin of coronary artery from the pulmonary artery

Anomalous origin of coronary artery from the pulmonary artery (ACAPA) is a rare congenital heart defect in which one or both coronary arteries arise from the pulmonary artery instead of the aorta. The most common and well-known subtype is anomalous left coronary artery from the pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome. Less frequently, the right coronary artery (ARCAPA) or both coronary arteries may originate from the pulmonary artery. Because the pulmonary artery carries deoxygenated blood at lower pressure, the affected coronary artery delivers inadequate oxygen to the heart muscle (myocardium), leading to myocardial ischemia and potentially life-threatening complications. In infants with ALCAPA, symptoms typically develop within the first few months of life as pulmonary vascular resistance drops after birth, reducing perfusion pressure in the anomalous coronary artery. Key clinical features include heart failure, poor feeding, irritability (especially during feeding or crying due to angina-like chest pain), excessive sweating, pallor, and failure to thrive. Left ventricular dysfunction and mitral regurgitation are common findings. Without treatment, ALCAPA carries a high mortality rate in infancy, with up to 90% of untreated infants dying within the first year of life. Some patients develop extensive collateral circulation between the right and left coronary systems, which may allow survival into childhood or adulthood, sometimes presenting with exercise intolerance, arrhythmias, or sudden cardiac death. ARCAPA tends to have a milder presentation and may be discovered incidentally. The primary treatment for ACAPA is surgical correction, which aims to establish a two-coronary-artery system with both arteries arising from the aorta. The most commonly performed procedure is direct reimplantation of the anomalous coronary artery into the aorta (the Takeuchi procedure or direct transfer technique). Early surgical intervention significantly improves outcomes, and most patients who undergo successful repair have good long-term prognosis with recovery of left ventricular function. Mitral valve repair may be performed concurrently if significant regurgitation is present. Lifelong cardiac follow-up is recommended to monitor for potential complications including coronary artery stenosis, arrhythmias, and residual ventricular dysfunction.

Common questions about Anomalous origin of coronary artery from the pulmonary artery